Rare Alveolar Diseases Flashcards

1
Q

A lung biopsy is shown below. The alveoli and alveolar ducts contain a granular eosinophilic material and very high surfactant content. What is the Dx? What is the likely cellular and molecular pathogenensis of this disease?

A

Alveolar proteinosis (Lipoproteinosis); Macrophages can’t clear surfactant, Defective activity/deficiency GM-CSF

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2
Q

What are important findings that point towards a Dx of alveolar proteinosis?

A
  • Heavy lungs with viscid yellow fluid
  • High conc. of surfactant
  • Architecture of lung intact with little inflammation
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3
Q

An adult patient presents with fever, productive cough and dyspnea. CXR shows diffuse bilateral symmetric alveolar infiltrates. The patient has a Hx of recurrent resp. tract infections. What is Dx?

A

Alveolar proteinosis

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4
Q

How are diffuse pulmonary hemorrhage syndromes characterized? What type of infiltrates are found in virtually all of these syndromes?

A

Either acute hemorrhagic or chronic hemorrhagic; Neutrophilic infiltrate (neutrophilic capillaritis)

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5
Q

A young man presents to the clinic with hemoptysis, dyspnea, weakness, and mild anemia. Lung biopsy is shown below. Biopsy reveals diffuse alveolar hemorrhage and hemosiderin-laden macrophages. The patient dies two years later. What is the Dx? What other Syx would the patient likely show? How are Dx generally made?

A

Goodpasture syndrome; Glomerulonephritis, circulating autoantibody to a component of basement membranes; Renal or pulmonary biopsy

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6
Q

A patient presents with glomerulonephritis and is found to have a P-ANCA against myeloperoxidase. Furthermore, the lung biopsy reveals alveolar hemorrhage with neutrophilic capillaritis. What is the Dx?

A

Microscopic polyangiitis

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7
Q

A patient presents with cough, dyspnea, substernal chest pain, fatigue, iron deficiency anemia but healthy renal function. The patient states that the Syx have been protracted. Lung biopsy shows diffuse alveolar bleeding. Dx?

A

Idiopathic Pulmonary Hemorrhage

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8
Q

What is the risk of giving cow’s milk to infants and children?

A

It can cause hypersensitivity to cow’s milk which results in diffuse pulmonary hemorrhage similar to IPH

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9
Q

A patient presents with fever, night sweats, weight loss, productive cough and dyspnea. The chest x-ray shows peripheral alveolar infiltrates with sparing of the hilum. A pulmonary biopsy is shown below. What is the Dx? What is the colloquial term for the CXR findings?

A

Eosinophilic penumonia; Photographic negative of pulmonary edema

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10
Q

What is found within the alveoli of a person with eosinophilic pneumonia?

A

Eosinophils, alveolar macrophages, proteinaceous exudate

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11
Q

What is simple eosinophilic pneumonia? What is another name for this condition?

A

Mild condition with fleeting pulmonary infiltrates that resolve in a month. Patients have peripheral blood eosinophilia but are often asymptomatic; Loeffler Syndrome

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12
Q

A patient with a severe strep throat infection is treated with antibiotics and anti-inflammatory agents. A few days later, the patient experiences pulmonary disease which recovers without long-term sequelae. What was the inciting agent and what can be found in the lungs and serum? Dx?

A

Secondary Eosinophilic Pneumonia of a drug hypersensitivity etiology; Drugs; Eosinophils

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13
Q

In temperate zones, what bug is most likely to cause secondary eosinophilic pneumonia?

A

Ascaris lumbricoides

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14
Q

In tropical zones, what bugs are most likely to cause secondary eosinophilic pneumonia?

A

The filarial nematodes Wuchereria bancrofti and Brugi malayi

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15
Q

A young, relatively healthy patient presents complaining of dyspnea and a lung biopsy is performed. On th biopsy, golden-yellow color is observed and macrophages with needle-shaped clefts are observed. What is the likely Dx?

A

Endogenous Lipid Pneumonia

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16
Q

An asymptomatic older patient has a CXR performed and an unexpected mass is found. They are relatively healthy claiming that their only medication is nose drops and laxatives. What is the likely Dx? Describe the morphology found on gross exam.

A

Exogenous Lipid Pneumonia due to aspirated oils; The oils are surrounded as a foreign body by granulomatous inflammation